Overview

  • Product name

  • Description

    Rabbit polyclonal to ZMPSTE24
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, ICC/IFmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse
  • Immunogen

    Recombinant fragment corresponding to Human ZMPSTE24 aa 217-347.
    Sequence:

    DYIAPLFDKFTPLPEGKLKEEIEVMAKSIDFPLTKVYVVEGSKRSSHSNA YFYGFFKNKRIVLFDTLLEEYSVLNKDIQEDSGMEPRNEEEGNSEEIKAK VKNKKQGCKNEEVLAVLGHELGHWKLGHTVK


    Database link: O75844

  • Positive control

    • IHC-P: Human liver and gastric cancer tissue. ICC/IF: PC-3 cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab234832 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
IHC-P 1/20 - 1/200.
ICC/IF 1/50 - 1/200.

Target

  • Function

    Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C.
  • Tissue specificity

    Widely expressed. High levels in kidney, prostate, testis and ovary.
  • Involvement in disease

    Defects in ZMPSTE24 are the cause of mandibuloacral dysplasia with type B lipodystrophy (MADB) [MIM:608612]. Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and types A or B patterns of lipodystrophy. Type B lipodystrophy observed in MADB, is characterized by generalized fat loss.
    Defects in ZMPSTE24 are a cause of lethal tight skin contracture syndrome (LTSCS) [MIM:275210]; also called restrictive dermopathy (RD). Lethal tight skin contracture syndrome is a rare disorder mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial features (small mouth, small pinched nose and micrognathia), sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life. The overall prevalence of consanguineous cases suggested an autosomal recessive inheritance.
  • Sequence similarities

    Belongs to the peptidase M48A family.
  • Cellular localization

    Endoplasmic reticulum membrane. Golgi apparatus membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • CAAX prenyl protease 1 homolog antibody
    • FACE-1 antibody
    • FACE1 antibody
    • FACE1_HUMAN antibody
    • Farnesylated proteins converting enzyme 1 antibody
    • Farnesylated proteins-converting enzyme 1 antibody
    • Prenyl protein specific endoprotease 1 antibody
    • Prenyl protein-specific endoprotease 1 antibody
    • STE24 antibody
    • Zinc metalloproteinase Ste24 homolog antibody
    • zmpste24 antibody
    see all

Images

  • Paraffin-embedded human gastric cancer tissue stained for ZMPSTE24 using ab234832 at 1/100 dilution in immunohistochemical analysis.

  • PC-3 (human prostate adenocarcinoma cell line) cells labeling ZMPSTE24 using ab234832 at 1/100 dilution in ICC/IF. Alexa Fluor® 488-conjugated Goat Anti-Rabbit IgG (H+L) was used as the secondary antibody.

  • Paraffin-embedded human liver tissue stained for ZMPSTE24 using ab234832 at 1/100 dilution in immunohistochemical analysis.

References

ab234832 has not yet been referenced specifically in any publications.

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